title: Long-term remission of pulmonary veno-occlusive disease associated with primary Sjögren's syndrome following immunosuppressive therapy.
abstract: The patient described here is a 21-year-old Japanese woman with primary Sjögren's syndrome (pSS) presenting with worsening of dyspnea, palpitation, recurrent parotitis, and arthritis. Chest computed tomography showed diffuse interlobular septal thickening and ground-glass opacities. Right heart catheterization demonstrated pulmonary hypertension, right-sided heart failure, normal pulmonary capillary wedge pressure, and no evidence of arterio-venous shunt. Transbronchial lung biopsy showed luminal obliteration of pulmonary venules by intimal cellular proliferations, without abnormalities in the small pulmonary arteries. These findings were consistent with pulmonary veno-occlusive disease (PVOD). Immunosuppressive therapy, starting with prednisolone 20 mg/day and subsequently combined with azathioprine, resulted in the disappearance of the signs and symptoms, including exertional dyspnea and abnormal pulmonary parenchymal shadows on computed tomography, and the normalization of pulmonary artery pressure. So far, there have been no reported cases of PVOD associated with pSS. Of interest, immunosuppressive therapy without vasodilator therapy almost completely resolved the pulmonary hypertension in this patient.
authors.affiliation: Division of Rheumatology, Nagoya City University Hospital, and Department of Medical Oncology and Immunology, Nagoya City University Graduate School of Medical Sciences, Kawasumi, Mizuho-ku, Nagoya, Aichi 467-8601, Japan. tnaniwa@med.nagoya-cu.ac.jp
authors.forename: Taio
authors.initials: T
authors.lastname: Naniwa
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Takeda
mesh_terms: D001379:Azathioprine / Q000627:therapeutic use*; D005260:Female; D006333:Heart Failure / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D011239:Prednisolone / Q000627:therapeutic use*; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D011859:Radiography; D012074:Remission Induction; D012129:Respiratory Function Tests; D012859:Sjogren's Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine